Whole body composition analysis by the BodPod air‐displacement plethysmography method in children with phenylketonuria shows a higher body fat percentage

Albersen, Monique; Bonthuis, Marjolein; Roos, N.M. de; Hurk, Dorine A. M. van den; Weber, E. Carbasius; Hendriks, Margriet M. W. B.; Velden, Monique G.M. de Sain–van der; Koning, Tom J. de; Visser, Gepke

doi:10.1007/s10545-010-9149-8

Cited by 37 publications

(54 citation statements)

References 14 publications

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“…Others have found correlations between (fat-free) muscle mass and protein intake (Huemer et al 2007). As in other studies (Albersen et al 2010;MacDonald et al 2011), we saw a tendency to obesity in our cohort.…”

Section: Mri Morphological Findingssupporting

confidence: 87%

Dietary Habits and Metabolic Control in Adolescents and Young Adults with Phenylketonuria: Self-Imposed Protein Restriction May Be Harmful

et al. 2013

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Background: In untreated patients, phenylketonuria (PKU) results in severe encephalopathy with mental retardation. A protein-restricted diet is recommended which can be relaxed in adolescence/adulthood.Methods: We contacted all 72 adult/adolescent PKU patients who had been treated in our center during early childhood. Some still regularly attended our outpatient clinics, while others were lost for follow-up, giving 51 patients in our study. We asked all patients to complete a dietary protocol as well as a questionnaire on quality of life. Blood and urine were analyzed and body impedance plethysmography and cerebral MRI were performed.Results: 42 % of the patients followed protein restriction supplemented with amino acid mixtures (AAM), others had a vegan diet with (8 %) or without (14 %) AAM; 36 % said they were eating normally and did not need any AAM. However, based on dietary protocols and blood urea levels, protein intake was restricted in this patient group. None of the patients examined had serious nutritional deficits. Phenylalanine levels were higher in patients not taking AAM. MRI of the brain was not different from those following protein restriction and taking AAM. The lesions score and mood correlated best with the cumulative phenylalanine values during the first 10 years of life.Conclusion: In summary, 50 % of adult/adolescent patients from our center did not take AAM at the start of our survey although they unknowingly followed selfimposed protein restriction. They had no overt nutritional deficits; however, long-term brain function may be compromised. Our study emphasizes the need for specialized metabolic care in PKU during adulthood.

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Section: Mri Morphological Findingssupporting

confidence: 87%

Dietary Habits and Metabolic Control in Adolescents and Young Adults with Phenylketonuria: Self-Imposed Protein Restriction May Be Harmful

et al. 2013

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“…Consistent with our findings, a study in male Wistar rats indicates that GMP limits expansion of fat mass while maintaining lean mass (49). Given the rising incidence of overweight in children and adults with PKU (3,33), further investigation is warranted to determine the relevance of our findings to the treatment and/or prevention of obesity in patients with PKU.…”

Section: E892 Gmp Diet Modifies Metabolic Phenotype Of Pku Micesupporting

confidence: 86%

“…Our studies in humans with PKU (30,38,54) indicate that GMP improves protein retention, phe concentrations, and satiety and palatability of the low-phe diet. However, questions remain about the ability of GMP to support long-term growth, physiological function, and energy balance in those with PKU, especially given the rising incidence of overweight in children and adults with PKU treated with conventional AA diets (3,33).…”

mentioning

confidence: 99%

Glycomacropeptide, a low-phenylalanine protein isolated from cheese whey, supports growth and attenuates metabolic stress in the murine model of phenylketonuria

Solverson

Murali²,

Brinkman³

et al. 2012

American Journal of Physiology-Endocrinology and Metabolism

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Solverson P, Murali SG, Brinkman AS, Nelson DW, Clayton MK, Yen CL, Ney DM. Glycomacropeptide, a low-phenylalanine protein isolated from cheese whey, supports growth and attenuates metabolic stress in the murine model of phenylketonuria. Am J Physiol Endocrinol Metab 302: E885-E895, 2012. First published January 31, 2012; doi:10.1152/ajpendo.00647.2011.-Phenylketonuria (PKU) is caused by a mutation in the phenylalanine (phe) hydroxylase gene and requires a low-phe diet plus amino acid (AA) formula to prevent cognitive impairment. Glycomacropeptide (GMP) contains minimal phe and provides a palatable alternative to AA formula. Our objective was to compare growth, body composition, and energy balance in Pah enu2 (PKU) and wild-type mice fed low-phe GMP, low-phe AA, or high-phe casein diets from 3-23 wk of age. The 2 ϫ 2 ϫ 3 design included main effects of genotype, sex, and diet. Fat and lean mass were assessed by dual-energy X-ray absorptiometry, and acute energy balance was assessed by indirect calorimetry. PKU mice showed growth and lean mass similar to wild-type littermates fed the GMP or AA diets; however, they exhibited a 3-15% increase in energy expenditure, as reflected in oxygen consumption, and a 3-30% increase in food intake. The GMP diet significantly reduced energy expenditure, food intake, and plasma phe concentration in PKU mice compared with the casein diet. The high-phe casein diet or the low-phe AA diet induced metabolic stress in PKU mice, as reflected in increased energy expenditure and intake of food and water, increased renal and spleen mass, and elevated plasma cytokine concentrations consistent with systemic inflammation. The low-phe GMP diet significantly attenuated these adverse effects. Moreover, total fat mass, %body fat, and the respiratory exchange ratio (CO 2 produced/O2 consumed) were significantly lower in PKU mice fed GMP compared with AA diets. In summary, GMP provides a physiological source of low-phe dietary protein that promotes growth and attenuates the metabolic stress induced by a high-phe casein or low-phe AA diet in PKU mice. indirect calorimetry; energy balance; splenomegaly; cytokines; amino acid metabolism

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“…However, studies in several other non-US populations have not confirmed the tendency for PKU patients to become overweight nor have they found inherent differences in resting energy expenditure that would explain an increased risk for overweight in PKU patients [11,12]. Although no difference in body composition was found in pediatric PKU patients relative to a control population in an Austrian study [11], a more recent study detected a higher fat mass in Dutch pediatric PKU patients relative to controls [13].…”

Section: Introductionmentioning

confidence: 92%

High prevalence of overweight and obesity in females with phenylketonuria

Burrage

McConnell

Haesler

et al. 2012

Molecular Genetics and Metabolism

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Whole body composition analysis by the BodPod air‐displacement plethysmography method in children with phenylketonuria shows a higher body fat percentage

Cited by 37 publications

References 14 publications

Dietary Habits and Metabolic Control in Adolescents and Young Adults with Phenylketonuria: Self-Imposed Protein Restriction May Be Harmful

Dietary Habits and Metabolic Control in Adolescents and Young Adults with Phenylketonuria: Self-Imposed Protein Restriction May Be Harmful

Glycomacropeptide, a low-phenylalanine protein isolated from cheese whey, supports growth and attenuates metabolic stress in the murine model of phenylketonuria

High prevalence of overweight and obesity in females with phenylketonuria

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